Expression of BCL6 in paediatric B-cell acute lymphoblastic leukaemia and association with prognosis.

Mäkinen A, Nikkilä A, Mehtonen J, Teppo S, Oksa L, Nordlund J, Rounioja S, Pohjolainen V, Laukkanen S, Heinäniemi M, Paavonen T, Lohi O

Pathology 53 (7) 875-882 [2021-12-00; online 2021-05-26]

B-cell lineage acute lymphoblastic leukaemia (B-ALL) is the most common paediatric malignancy. Transcription factor B-cell lymphoma 6 (BCL6) is essential to germinal centre formation and antibody affinity maturation and plays a major role in mature B-cell malignancies. More recently, it was shown to act as a critical downstream regulator in pre-BCR+ B-ALL. We investigated the expression of the BCL6 protein in a population-based cohort of paediatric B-ALL cases and detected moderate to strong positivity through immunohistochemistry in 7% of cases (8/117); however, only two of eight BCL6 cases (25%) co-expressed the ZAP70 protein. In light of these data, the subtype with active pre-BCR signalling constitutes a rare entity in paediatric B-ALL. In three independent larger cohorts with gene expression data, high BCL6 mRNA levels were associated with the TCF3-PBX1, Ph-like, NUTM1, MEF2D and PAX5-alt subgroups and the 'metagene' signature for pre-BCR-associated genes. However, higher-than-median BCL6 mRNA level alone was associated with favourable event free survival in the Nordic paediatric cohort, indicating that using BCL6 as a diagnostic marker requires careful design, and evaluation of protein level is needed alongside the genetic or transcriptomic data.

NGI Uppsala (SNP&SEQ Technology Platform)

National Genomics Infrastructure

PubMed 34049715

DOI 10.1016/j.pathol.2021.02.013

Crossref 10.1016/j.pathol.2021.02.013

pii: S0031-3025(21)00109-4


Publications 7.2.7